Description
Product Characteristics:
ACADVL (acyl-Coenzyme A dehydrogenase, very long chain) catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. It is specific to esters of long-chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA. Deficiencies in ACADVL are associated with reduced myocardial fatty acid beta-oxidation and cardiomyopathy.
Subcellular location: Cytoplasm, Cell membrane
Synonyms: ACAD 6, ACAD6, ACADV_HUMAN, Acadvl, Acyl CoA dehydrogenase very long chain, Acyl Coenzyme A dehydrogenase very long chain, LCACD, mitochondrial, Very long chain specic acyl CoA dehydrogenase, Very long chain specic acyl CoA dehydrogenase mitochondrial, Very long-chain specic acyl-CoA dehydrogenase, VLCAD.
Target Information: The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]